Tumors

and Pilar González-Peramato²

(1)

Department of Anatomy, Histology and Neuroscience, Universidad Autónoma de Madrid, Madrid, Spain

(2)

Department of Pathology, University Hospital La Paz, Universidad Autónoma de Madrid, Madrid, Spain

6.1 Lymphoma

6.2 Plasmacytoma

6.3 Other Tumors

Keywords

Testicular neoplasmsPrimary testicular lymphomaPrimary testicular plasmacytomaTesticular interdigitating dendritic cell tumor

The existence of primary lymphomas of the testis is a controversial fact. It is true that in some cases testicular involvement is the only manifestation of a lymphoma, but it is also true that most cases spread rapidly, suggesting that they may have been the clinical manifestation of an occult lymphoma. The selected cases are a follicular lymphoma in the pediatric age due to the peculiar expression of Bcl6 and two lymphomas in adulthood. A testicular plasmacytoma and an interdigitating dendritic cell tumor are also included in this chapter.

6.1 Lymphoma

Case 93. Primary Follicular Lymphoma of the Pediatric Testis

Clinical case. A 3-year-old boy with an enlarged left testicle. The ultrasound showed a hypoechoic nodule 1.8 cm in diameter. Serum levels of testicular tumor markers were negative.

Pathological findings. A well-defined grayish tumor that occupies most of the testicular parenchyma. The tumor appears to be well demarcated from the rest of the parenchyma. Histologically, a multinodular structure with a tendency to fusion of the different nodules is appreciated. The central part of the nodules is formed by a proliferation of cells similar to the centroblasts mixed with some centrocytes. These cells are CD20, CD10, and Bcl6 positive and negative for Bcl2, CD23. The proliferation index (Ki67) is higher than 60%. The peripheral interfollicular cells are T lymphocytes and reactive dendritic cells. The tumor infiltrates neither the albuginea, nor the vessels or the paratesticular structures.

../images/471462_1_En_6_Chapter/471462_1_En_6_Figa_HTML.jpg — Fig. 1

The testicular parenchyma is partially replaced by a lymphoid proliferation of nodular pattern. The demarcation with the seminiferous tubules is clear

../images/471462_1_En_6_Chapter/471462_1_En_6_Figb_HTML.jpg — Fig. 2

The central part of the nodule is made up of cells similar to centroblasts with some centrocytes among them

../images/471462_1_En_6_Chapter/471462_1_En_6_Figc_HTML.jpg — Fig. 3

Most cells of the nodules express CD20. Some have advanced into the intertubular interstitium

../images/471462_1_En_6_Chapter/471462_1_En_6_Figd_HTML.jpg — Fig. 4

Many cells in the central part of the nodules are positive for Bcl6

../images/471462_1_En_6_Chapter/471462_1_En_6_Fige_HTML.jpg — Fig. 5

The cells in the center of the follicle lacks immunoexpression for Bcl2

../images/471462_1_En_6_Chapter/471462_1_En_6_Figf_HTML.jpg — Fig. 6

Intense immunostaining for CD10 in the cells of the central part of the nodules

../images/471462_1_En_6_Chapter/471462_1_En_6_Figg_HTML.jpg — Fig. 7

Intense immunostaining for CD31 of the peripheral crown of the nodules and to a lesser extent in the center of the follicle

../images/471462_1_En_6_Chapter/471462_1_En_6_Figh_HTML.jpg — Fig. 8

Absence of immunostaining for CD23 in the cells in the center of the nodules

../images/471462_1_En_6_Chapter/471462_1_En_6_Figi_HTML.jpg — Fig. 9

The proliferation index estimated with Ki67 is very high in the cells of the center of the follicles

Comments. Pediatric follicular lymphomas are considered a variety distinct from adult follicular lymphomas. The histology of pediatric testicular lymphomas is similar to that of adult large cell B-cell lymphomas. Immunohistochemically, the most interesting datum is that the tumor cells express Bcl6. This fact suggests that there are two different tumors. In pediatric lymphomas, the translocation between the immunoglobulin heavy chain on chromosome 14 and the Bcl2 gene on chromosome 18 has not been observed, so they do not have Bcl2 overexpression. On the other hand, none of the pediatric follicular lymphomas expresses p53 protein, which frequently occurs in adult follicular lymphomas. And finally, the prognosis is excellent.

References

1.

Pileri SA, Sabattini E, Rosito P, Zinzani PL, Ascani S, Fraternali-Orcioni G, Gamberi B, Piccioli M, Vivenza D, Falini B, Gaidano G. Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics. J Clin Pathol. 2002;55:684–8.
2.

Heller KN, Teruya-Feldstein J, La Quaglia MP, Wexler LH. Primary follicular lymphoma of the testis: excellent outcome following surgical resection without adjuvant chemotherapy. J Pediatr Hematol Oncol. 2004;26:104–7.
3.

Lones MA, Raphael M, McCarthy K, Wotherspoon A, Terrier-Lacombe MJ, Ramsay AD, Maclennan K, Cairo MS, Gerrard M, Michon J, Patte C, Pinkerton R, Sender L, Auperin A, Sposto R, Weston C, Heerema NA, Sanger WG, von Allmen D, Perkins SL. Primary follicular lymphoma of the testis in children and adolescents. J Pediatr Hematol Oncol. 2012;34:68–71.
4.

Liu Q, Salaverria I, Pittaluga S, Jegalian AG, Xi L, Siebert R, Raffeld M, Hewitt SM, Jaffe ES. Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma. Am J Surg Pathol. 2013;37:333–43.

Case 94. Primary Diffuse Large B-Cell Lymphoma Germinal Center Phenotype

Cinical case. A 29-year-old patient who consulted due to a voluminous ulcerated mass in his left testicle since several months ago, that did not remit with antibiotic therapy. The Doppler ultrasound revealed a heterogeneous mass with flow in which it is difficult to distinguish the testicle form what is suspected to be a testicular tumor. Testicular tumor markers were negative. Left hemiscrotectomy, removal of the mass and four identifiable cm of spermatic cord was performed.

Pathological findings. The surgical specimen weighs 512 g. It is partially covered by skin with a 2.5 × 1.8-cm ulceration. It measures 14 × 18 cm and the spermatic cord is recognized. The sectioned surface is gray with partially necrotic nodular areas. No testicle or epididymis is identified. A tissue with similar characteristics goes to the skin and to all the surgical margins, except for the end of the spermatic cord. With the specimen, 7 lymph nodes of the inguinal region are remitted, the largest one being 2.2 cm in maximum diameter. The tumor is composed entirely of medium to large lymphoid cells that completely destroy the testicular parenchyma, epididymis, and extend to paratesticular soft tissues and the initial part of the spermatic cord. A striking affectation of venous structures with destruction of its wall is observed. The percentage of necrosis is approximately 35% of the tumor. The immunophenotype is as follows: intensely positive for CD45, CD20, and CD10. Moderately positive in a diffuse pattern for Bcl6. The following markers have been negative: CD138, CD3, CD30, Bcl2, ALK, NK markers (CD56, TDT, perforin, granzyme), EBER, histiocytic markers-dendritic cells (CD68, CD23, and CD21), and MUM1. The proliferative index (Ki67) is 85% of the tumor cells. Only two lymph nodes showed focal involvement. FISH study for Bcl2 and cMYC translocation is negative.